CTD Flashcards
nephritic syndrome - kidney involvement in SLE
heavy protein, hypoalbuminaemia and peripheral oedema
acute nephritic syndrome comprises of haematuria, proteinuria, HTN, oedema, oliguria and uraemia
symptoms: haematuira, proteinuria, oedema, weight gain, HTN
caused by lupus
presentation is highly variable
suspected SLE questions in a history
raynauds hair loss mouth ulcers fatigue weigh tloss systems review - cardiorespiratory and GI/GU symptoms
investigations in suspected lupus
FBC
ESR and CRP - CRP generally stays normal in SLE and ESR goes up with active SLE (if CRP is elevated exclude infection)
U&Es
ANA - screening test for CTD, negative result excludes SLE
LFTs- albumin (low in nephritic syndrome)
ENAs
dsDNA
compliment levels (C3/4)- low when disease is active
24 hour urinary protein or protein creatinine ratio
MSU looking for casts
symptoms of SLE
low grade fever skin is photosensitive mouth ulcers aching muscles arthritis in joints fatigue loss of appetite mallor rash pleura and pericardium inflammation raynauds
pregnancy and lupus
should be planned
including ensuring immunosuppressants are changed if existing regime isn’t safe in pregnancy
presence of Ro and La and antiphospholipid antibodies should be known
Ro and La can cross placenta and result in neonatal lupus can include rash, complete heart block and blood abnormalities
use POP and physical barrier methods - oestrogen can make the disease more likely to flare up
antiphospholipid syndrome
elevated antiphospholipid antibodies and an acquired thrombophillia or clotting tendency
diagnosis: positive antiphospholipid antibodies on two or more occasions at least 12 weeks apart and vascular thrombosis or pregnancy morbidity
diagnosis of SLE
4 or more of criteria
malor rash discoid rash photosensitivity oral ulcers non-erosive arthritis pleurites or pericarditis renal disorder neurological disorder - seizure or psychosis haematological disorder immunologcal disorder - antibodies dsDNA, SM, others positive ANA
management of SLE
maintenance
short courses of NSAIDs for symptomatic control
prednisone at a low dose may be required for maintenance therapy
avoid the sun and use high protection factor
hydroxychloroquine for joint and skin symptoms
azathioprine, methotrexate and mycophenolate - steroid sparing agent
severe flare: high dose prednisone and IV cyclophosphamide (suppress immune system)
sjorgens
chronic inflammatory autoimmune disorder
can be associated with RA, SLE, systemic sclerosis
lymphocytic infiltration and fibrosis of exocrine glands, especially lacrimal and salivary glands
decreased tear production, salivation, parotid swelling., vaginal dryness, dry cough, dysphagia
schirmers test measures conjunctival production
systemic sclerosis
skin fibrosis and vascular disease
limited cutaneous aka CREST - skin involvement limited to the face hands and feet
diffuse cutaneous whole body in severe cases early organ fibrosis annual echo and spirometry prognosis poor
polymyositis and dermatomyositis
proximal muscle weakness and auto immune mediated striated muscle inflammation associated with pain in muscles and joints
dermatomyositis
also skin involvement
heliotrope rash - purple one
lots more
